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There are 5512 results for: content related to: Unravelling the twists and turns of the serpinopathies

  1. You have free access to this content
    A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with α1-antitrypsin deficiency


    Volume 52, Issue 3, September 2010, Pages: 1078–1088, Elena Miranda, Juan Pérez, Ugo I. Ekeowa, Nedim Hadzic, Noor Kalsheker, Bibek Gooptu, Bernard Portmann, Didier Belorgey, Marian Hill, Susan Chambers, Jeff Teckman, Graeme J. Alexander, Stefan J. Marciniak and David A. Lomas

    Article first published online : 14 MAY 2010, DOI: 10.1002/hep.23760

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    pH-dependent stability of neuroserpin is mediated by histidines 119 and 138; Implications for the control of β-sheet A and polymerization

    Protein Science

    Volume 19, Issue 2, February 2010, Pages: 220–228, Didier Belorgey, Peter Hägglöf, Maki Onda and David A. Lomas

    Article first published online : 1 DEC 2009, DOI: 10.1002/pro.299

  3. You have free access to this content
    Neuroserpin Portland (Ser52Arg) is trapped as an inactive intermediate that rapidly forms polymers

    European Journal of Biochemistry

    Volume 271, Issue 16, August 2004, Pages: 3360–3367, Didier Belorgey, Lynda K. Sharp, Damian C. Crowther, Maki Onda, Jan Johansson and David A. Lomas

    Article first published online : 23 JUL 2004, DOI: 10.1111/j.1432-1033.2004.04270.x

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    Mutagenesis of the bovSERPINA3-3 demonstrates the requirement of aspartate-371 for intermolecular interaction and formation of dimers

    Protein Science

    Volume 21, Issue 7, July 2012, Pages: 977–986, X. Blanchet, A. Péré-Brissaud, N. Duprat, E. Pinault, D. Delourme, A. Ouali, C. Combet, A. Maftah, P. Pélissier and L. Brémaud

    Article first published online : 18 MAY 2012, DOI: 10.1002/pro.2078

  5. Inhibiting Pathogenic Protein Aggregation: Combinatorial Chemistry in Combating Alpha-1 Antitrypsin Deficiency

    New Strategies in Chemical Synthesis and Catalysis

    Bruno Pignataro, Pages: 297–324, 2012

    Published Online : 17 APR 2012, DOI: 10.1002/9783527645824.ch13

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    Sugar and alcohol molecules provide a therapeutic strategy for the serpinopathies that cause dementia and cirrhosis

    FEBS Journal

    Volume 273, Issue 11, June 2006, Pages: 2450–2552, Lynda K. Sharp, Meera Mallya, Kerri J. Kinghorn, Zhen Wang, Damian C. Crowther, James A. Huntington, Didier Belorgey and David A. Lomas

    Article first published online : 8 MAY 2006, DOI: 10.1111/j.1742-4658.2006.05262.x

  7. You have full text access to this OnlineOpen article
    Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiency


    Volume 57, Issue 5, May 2013, Pages: 2049–2060, Adriana Ordóñez, Erik L. Snapp, Lu Tan, Elena Miranda, Stefan J. Marciniak and David A. Lomas

    Article first published online : 14 MAR 2013, DOI: 10.1002/hep.26173

  8. You have free access to this content
    Molecular pathogenesis of liver disease in α1-antitrypsin deficiency


    Volume 24, Issue 6, December 1996, Pages: 1504–1516, J H Teckman, D Qu and D H Perlmutter

    Article first published online : 30 DEC 2003, DOI: 10.1002/hep.510240635

  9. On the molecular structure of human neuroserpin polymers

    Proteins: Structure, Function, and Bioinformatics

    Volume 80, Issue 1, January 2012, Pages: 8–13, Maria Grazia Santangelo, Rosina Noto, Matteo Levantino, Antonio Cupane, Stefano Ricagno, Margherita Pezzullo, Martino Bolognesi, Maria Rosalia Mangione, Vincenzo Martorana and Mauro Manno

    Article first published online : 9 NOV 2011, DOI: 10.1002/prot.23197

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    Serpin structure, function and dysfunction

    Journal of Thrombosis and Haemostasis

    Volume 9, Issue s1, July 2011, Pages: 26–34, J. A. HUNTINGTON

    Article first published online : 22 JUL 2011, DOI: 10.1111/j.1538-7836.2011.04360.x

  11. Alpha-1-Antitrypsin Deficiency

    Protein Misfolding Diseases: Current and Emerging Principles and Therapies

    David A. Lomas, David H. Perlmutter, Pages: 403–423, 2010

    Published Online : 2 JUL 2010, DOI: 10.1002/9780470572702.ch18

  12. Familial conformational diseases and dementias

    Human Mutation

    Volume 20, Issue 1, July 2002, Pages: 1–14, Damian C. Crowther

    Article first published online : 27 JUN 2002, DOI: 10.1002/humu.10100

  13. You have free access to this content
    Topography of a 2.0 Å structure of α1-antitrypsin reveals targets for rational drug design to prevent conformational disease

    Protein Science

    Volume 9, Issue 7, 2000, Pages: 1274–1281, Peter R. Elliott, Xue Y. Pei, Timothy R. Dafforn and David A. Lomas

    Article first published online : 31 DEC 2008, DOI: 10.1110/ps.9.7.1274

  14. Encephalopathy with Neuroserpin Inclusion Bodies Presenting as Progressive Myoclonus Epilepsy and Associated with a Novel Mutation in the Proteinase Inhibitor 12 Gene

    Brain Pathology

    Volume 21, Issue 5, September 2011, Pages: 575–582, Matthew C. Hagen, Jill R. Murrell, Marie-Bernadette Delisle, Eva Andermann, Frederick Andermann, Marie Christine Guiot and Bernardino Ghetti

    Article first published online : 24 MAR 2011, DOI: 10.1111/j.1750-3639.2011.00481.x

  15. α1-Antitrypsin Deficiency

    Chronic Obstructive Pulmonary Disease

    Loutfi S. Aboussouan, James K. Stoller, Pages: 447–459, 2008

    Published Online : 11 JAN 2008, DOI: 10.1002/9780470755976.ch38

  16. Broad Spectrum of Hepatocyte Inclusions in Humans, Animals, and Experimental Models

    Standard Article

    Comprehensive Physiology

    Pavel Strnad, Renwar Nuraldeen, Nurdan Guldiken, Daniel Hartmann, Vineet Mahajan, Helmut Denk and Johannes Haybaeck

    Published Online : 22 OCT 2013, DOI: 10.1002/cphy.c120032

  17. You have full text access to this Open Access content
    Small-molecule peptides inhibit Z α1-antitrypsin polymerization

    Journal of Cellular and Molecular Medicine

    Volume 13, Issue 8b, August 2009, Pages: 2304–2316, Yi-Pin Chang, Ravi Mahadeva, Wun-Shaing W. Chang, Sheng-Chieh Lin and Yen-Ho Chu

    Article first published online : 16 DEC 2008, DOI: 10.1111/j.1582-4934.2008.00608.x

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    Type II antithrombin deficiency caused by a large in-frame insertion: structural, functional and pathological relevance

    Journal of Thrombosis and Haemostasis

    Volume 10, Issue 9, September 2012, Pages: 1859–1866, I. MARTÍNEZ-MARTÍNEZ, D. J. D. JOHNSON, M. YAMASAKI, J. NAVARRO-FERNÁNDEZ, A. ORDÓÑEZ, V. VICENTE, J. A. HUNTINGTON and J. CORRAL

    Article first published online : 4 SEP 2012, DOI: 10.1111/j.1538-7836.2012.04839.x

  19. You have free access to this content
    Induction of α1-antitrypsin synthesis in human articular chondrocytes by interleukin-6–type cytokines: Evidence for a local acute-phase response in the joint

    Arthritis & Rheumatism

    Volume 42, Issue 9, September 1999, Pages: 1936–1945, Dagmar-Christiane Fischer, Barbara Siebertz, Eddy Van De Leur, Karl-Heinz Schiwy-Bochat, Lutz Graeve, Peter-C. Heinrich and Hans-Dieter Haubeck

    Article first published online : 26 APR 2001, DOI: 10.1002/1529-0131(199909)42:9<1936::AID-ANR20>3.0.CO;2-K

  20. You have free access to this content
    Serpins 2005 – fun between the β-sheets

    FEBS Journal

    Volume 272, Issue 19, October 2005, Pages: 4868–4873, James C. Whisstock, Stephen P. Bottomley, Phillip I. Bird, Robert N. Pike and Paul Coughlin

    Article first published online : 16 SEP 2005, DOI: 10.1111/j.1742-4658.2005.04927.x